U.S. Department of Health & Human Services Divider Arrow National Institutes of Health Divider Arrow NCATS

Details

Stereochemistry ACHIRAL
Molecular Formula C2H7NS
Molecular Weight 77.149
Optical Activity NONE
Defined Stereocenters 0 / 0
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of CYSTEAMINE

SMILES

NCCS

InChI

InChIKey=UFULAYFCSOUIOV-UHFFFAOYSA-N
InChI=1S/C2H7NS/c3-1-2-4/h4H,1-3H2

HIDE SMILES / InChI

Molecular Formula C2H7NS
Molecular Weight 77.149
Charge 0
Count
Stereochemistry ACHIRAL
Additional Stereochemistry No
Defined Stereocenters 0 / 0
E/Z Centers 0
Optical Activity NONE

Description
Curator's Comment: description was created based on several sources, including https://www.ncbi.nlm.nih.gov/pubmed/6131501 | https://www.ncbi.nlm.nih.gov/pubmed/6124307 | https://www.ncbi.nlm.nih.gov/pubmed/15675041 | https://www.ncbi.nlm.nih.gov/pubmed/28384851

Cysteamine (trade name CYSTAGON) is a cystine-depleting agent indicated for the treatment of corneal cystine crystal accumulation in patients with cystinosis. Cystinosis is an autosomal recessive inborn error of metabolism in which the transport of cystine out of lysosomes is abnormal; in the nephropathic form, accumulation of cystine and formation of crystals damage various organs, especially the kidney, leading to renal tubular Fanconi Syndrome and progressive glomerular failure, with end-stage renal failure by the end of the first decade of life. In four studies of cystinosis patients before cysteamine was available, renal death (need for transplant or dialysis) occurred at the median age of fewer than 10 years. Patients with cystinosis also experience growth failure, rickets, and photophobia due to cystine deposits in the cornea. With time most organs are damaged, including the retina, muscles and central nervous system. Cysteamine is an aminothiol that participates within lysosomes in a thiol-disulfide interchange reaction converting cystine into cysteine and cysteine-cysteamine mixed disulfide, both of which can exit the lysosome in patients with cystinosis.

Approval Year

Targets

Targets

Primary TargetPharmacologyConditionPotency
Target ID: P21980
Gene ID: 7052.0
Gene Symbol: TGM2
Target Organism: Homo sapiens (Human)
178.0 µM [IC50]
Conditions

Conditions

ConditionModalityTargetsHighest PhaseProduct
Primary
CYSTAGON

Approved Use

PROCYSBI is indicated for the treatment of nephropathic cystinosis in adult and pediatric patients 2 years of age and older. PROCYSBI is a cystine-depleting agent indicated for the treatment of nephropathic cystinosis in adult and pediatric patients 2 years of age and older. (1)

Launch Date

1994
Cmax

Cmax

ValueDoseCo-administeredAnalytePopulation
37.72 μM
450 mg single, oral
dose: 450 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
2.7 mg/L
404 mg/m² 4 times / day steady-state, oral
dose: 404 mg/m²
route of administration: Oral
experiment type: STEADY-STATE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
AUC

AUC

ValueDoseCo-administeredAnalytePopulation
96 μM × h
450 mg single, oral
dose: 450 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
351 mg × min/L
404 mg/m² 4 times / day steady-state, oral
dose: 404 mg/m²
route of administration: Oral
experiment type: STEADY-STATE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
T1/2

T1/2

ValueDoseCo-administeredAnalytePopulation
90 min
404 mg/m² 4 times / day steady-state, oral
dose: 404 mg/m²
route of administration: Oral
experiment type: STEADY-STATE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
Funbound

Funbound

ValueDoseCo-administeredAnalytePopulation
48%
450 mg single, oral
dose: 450 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
48%
404 mg/m² 4 times / day steady-state, oral
dose: 404 mg/m²
route of administration: Oral
experiment type: STEADY-STATE
co-administered:
CYSTEAMINE plasma
Homo sapiens
population: UNHEALTHY
age: CHILD
sex: FEMALE / MALE
food status: UNKNOWN
Doses

Doses

DosePopulationAdverse events​
0.44 % 1 times / day multiple, ophthalmic
Recommended
Dose: 0.44 %, 1 times / day
Route: ophthalmic
Route: multiple
Dose: 0.44 %, 1 times / day
Sources:
unhealthy, 13.4 (5.9-27.8)
Health Status: unhealthy
Age Group: 13.4 (5.9-27.8)
Sex: M+F
Sources:
Disc. AE: Intracranial hypertension...
AEs leading to
discontinuation/dose reduction:
Intracranial hypertension
Sources:
600 mg 1 times / day multiple, oral
Recommended
Dose: 600 mg, 1 times / day
Route: oral
Route: multiple
Dose: 600 mg, 1 times / day
Sources:
healthy, 37.5 (19-64)
Health Status: healthy
Age Group: 37.5 (19-64)
Sex: M+F
Sources:
Disc. AE: Blurred vision, Hematuria...
AEs leading to
discontinuation/dose reduction:
Blurred vision
Hematuria
Sources:
AEs

AEs

AESignificanceDosePopulation
Intracranial hypertension Disc. AE
0.44 % 1 times / day multiple, ophthalmic
Recommended
Dose: 0.44 %, 1 times / day
Route: ophthalmic
Route: multiple
Dose: 0.44 %, 1 times / day
Sources:
unhealthy, 13.4 (5.9-27.8)
Health Status: unhealthy
Age Group: 13.4 (5.9-27.8)
Sex: M+F
Sources:
Blurred vision Disc. AE
600 mg 1 times / day multiple, oral
Recommended
Dose: 600 mg, 1 times / day
Route: oral
Route: multiple
Dose: 600 mg, 1 times / day
Sources:
healthy, 37.5 (19-64)
Health Status: healthy
Age Group: 37.5 (19-64)
Sex: M+F
Sources:
Hematuria Disc. AE
600 mg 1 times / day multiple, oral
Recommended
Dose: 600 mg, 1 times / day
Route: oral
Route: multiple
Dose: 600 mg, 1 times / day
Sources:
healthy, 37.5 (19-64)
Health Status: healthy
Age Group: 37.5 (19-64)
Sex: M+F
Sources:
OverviewDrug as perpetrator​

Drug as perpetrator​

TargetModalityActivityMetaboliteClinical evidence
inconclusive
inconclusive
inconclusive
no
no
no
no
no
no
no
no
no
no
no
no
no
no
no
weak [Inhibition 780 uM]
weak [Inhibition 780 uM]
Drug as victim

Drug as victim

TargetModalityActivityMetaboliteClinical evidence
no
no
no
no
no
no
no
no
yes
yes
yes
yes
yes
yes
yes
yes
yes
PubMed

PubMed

TitleDatePubMed
Electric potential control of DNA immobilization on gold electrode.
2003-01
In vivo confocal microscopy of the cornea in nephropathic cystinosis.
2002-12
Glutathione in gingival crevicular fluid and its relation to local antioxidant capacity in periodontal health and disease.
2002-12
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells.
2002-12
Cysteamine pre-treatment reduces pentylenetetrazol-induced plasticity and epileptiform discharge in the CA1 region of rat hippocampal slices.
2002-11-15
Characterization of the surfactin synthetase C-terminal thioesterase domain as a cyclic depsipeptide synthase.
2002-11-12
Nephropathic cystinosis associated with cardiomyopathy: a 27-year clinical follow-up.
2002-11-09
Possible mechanism for involvement of cysteine in aroma production in wine.
2002-10-09
Follow-up and treatment of adults with cystinosis in the Netherlands.
2002-10
Identification and quantitation of key aroma compounds formed in Maillard-type reactions of fructose with cysteamine or isothiaproline (1,3-thiazolidine-2-carboxylic acid).
2002-09-11
Structural determination and odor characterization of N-(2-mercaptoethyl)-1,3-thiazolidine, a new intense popcorn-like-smelling odorant.
2002-09-11
Studies on the anti-inflammatory and anti-nociceptive effects of melatonin in the rat.
2002-09
The ability of hippocampal CA1 area for induction of long-term potentiation is persistently reduced by prior treatment with cysteamine: an in vitro study.
2002-08
Differentiating the orientations of photosynthetic reaction centers on Au electrodes linked by different bifunctional reagents.
2002-08
Cystinosis.
2002-07-11
Synthesis of (+/-)-madindolines and chemical models. Studies of chemical reactivity.
2002-07-11
A molecular CT blood pool contrast agent.
2002-07
Cysteine-mediated electron transfer in syntrophic acetate oxidation by cocultures of Geobacter sulfurreducens and Wolinella succinogenes.
2002-07
The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function.
2002-06-13
Gastroprotective effect of adrenomedullin administered subcutaneously in the rat.
2002-06
X-ray crystallographic studies on butyryl-ACP reveal flexibility of the structure around a putative acyl chain binding site.
2002-06
Cysteamine supplementation during in vitro maturation and embryo culture: a useful tool for increasing the efficiency of bovine in vitro embryo production.
2002-06
Tumour targeting of humanised cross-linked divalent-Fab' antibody fragments: a clinical phase I/II study.
2002-05-06
Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome.
2002-05
Properties of selected S-nitrosothiols compared to nitrosylated WR-1065.
2002-05
Endogenous somatostatin inhibits interaction of insulin and cholecystokinin on exocrine secretion of isolated, perfused rat pancreas.
2002-05
Effect of sperm survival and CTC staining pattern on in vitro fertilization of porcine oocytes.
2002-04-15
Generation of roasted notes based on 2-acetyl-2-thiazoline and its precursor, 2-(1-hydroxyethyl)-4,5-dihydrothiazole, by combined bio and thermal approaches.
2002-04-10
Electrochemical and spectroelectrochemical behavior of the main photodegradation product of nifedipine: the nitrosopyridine derivative.
2002-04
Modulation of inflammatory paw oedema by cysteamine in the rat.
2002-04
Effects of tea from Turnera ulmifolia L. on mouse gastric mucosa support the Turneraceae as a new source of antiulcerogenic drugs.
2002-04
Subunit modification and association in VR1 ion channels.
2002-03-22
Prevalence of apoptosis and inner cell allocation in bovine embryos cultured under different oxygen tensions with or without cysteine addition.
2002-03-15
Effect of glutathione synthesis stimulation during in vitro maturation of ovine oocytes on embryo development and intracellular peroxide content.
2002-03-15
Polymer-cysteamine conjugates: new mucoadhesive excipients for drug delivery?
2002-03-02
Age-related prevalence of anterior segment complications in patients with infantile nephropathic cystinosis.
2002-03
Generating favorable nano-environments for thermal and solvent stabilization of immobilized beta-galactosidase.
2002-02-15
Evidence for a central inhibitory role of growth hormone secretagogues and ghrelin on gastric acid secretion in conscious rats.
2002-02
Role of porphyrin sequestration in the biogenesis of iron-laden astrocytic inclusions in primary culture.
2002
Role of heme oxygenase-1 in the biogenesis of corpora amylacea.
2002
A novel strategy for the synthesis of neoglycoconjugates from deacylated deep rough lipopolysaccharides.
2002
In vitro maturation and glutathione synthesis of porcine oocytes in the presence or absence of cysteamine under different oxygen tensions: role of cumulus cells.
2002
Interventions for paracetamol (acetaminophen) overdoses.
2002
Enhancement of solar inactivation of Escherichia coli by titanium dioxide photocatalytic oxidation.
2002
Gastric antisecretory and antiulcer activity of oxytocin in rats and guinea pigs.
2001-11-21
Adsorption properties and activities of lipase on a gold substrate modified by self-assembled monolayers.
2001-11
Synthesis and anti-melanoma activity of analogues of N-acetyl-4-S-cysteaminylphenol substituted with two methyl groups alpha to the nitrogen.
2001-02
Taurine and hypotaurine in spermatozoa and epididymal fluid of cats.
2001
Oxidative stress, glucose-6-phosphate dehydrogenase and the red cell.
2001
Cholesterol 7alpha-hydroxylase. 2. Biochemical properties and participation of endogenous cholesterol in the assay in vitro.
1975-06-16
Patents

Sample Use Guides

The recommended maintenance dose of 1.30 grams/m2/day. Patients over age 12 and over 110 pounds should receive 2.0 grams/day given in four divided doses as a starting maintenance dose. This dose should be reached after 4 to 6 weeks of incremental dosage increases as stated above. The dose should be raised if the leukocyte cystine level remains > 2 nmol/1⁄2 cystine/mg/protein.
Route of Administration: Oral
Oocytes were obtained from 4-6 weeks old Naval Medical Research Institute (NMRI) female mice, 48 hours after stimulation with Intraperitoneal (IP) injection of 10 IU Pregnant Mare Serum Gonadotropin (PMSG). Germinal Vesicle (GV) oocyte with and without cumulus cells were isolated from ovaries and cultured in Tissue Culture Medium (TCM) 199 with availability of 100 μM of CYS (Cysteamine). After 24 hours, mature oocyte in metaphase II (MII) were fertilized with sperm in In vitro Fertilization (IVF) medium (T6) and evaluated for fetal development into blastocyst. CYS could significantly (p<0.05) increase the rate of IVM and oocyte evolution, and embryo formation in medium culture.
Substance Class Chemical
Created
by admin
on Mon Mar 31 18:18:09 GMT 2025
Edited
by admin
on Mon Mar 31 18:18:09 GMT 2025
Record UNII
5UX2SD1KE2
Record Status Validated (UNII)
Record Version
  • Download
Name Type Language
MERCAPTAMINE
INN   MART.   WHO-DD  
INN  
Preferred Name English
CYSTEAMINE
HSDB   MI   USAN   VANDF  
USAN   INCI  
Official Name English
ETHANETHIOL, 2-AMINO-
Systematic Name English
CYSTEAMINE [MI]
Common Name English
CYSTEAMINE [USAN]
Common Name English
NSC-647528
Code English
CYSTEAMINE [VANDF]
Common Name English
MEA
Common Name English
mercaptamine [INN]
Common Name English
MERCAPTAMINE [MART.]
Common Name English
2-Aminoethanethiol
Systematic Name English
L-1573
Code English
CYSTEAMINE [HSDB]
Common Name English
2-MERCAPTOETHYLAMINE
Systematic Name English
Mercaptamine [WHO-DD]
Common Name English
Classification Tree Code System Code
LIVERTOX NBK548701
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
WHO-ATC S01XA21
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
FDA ORPHAN DRUG 231006
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
NCI_THESAURUS C29701
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
NDF-RT N0000175544
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
WHO-VATC QS01XA21
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
FDA ORPHAN DRUG 404613
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
WHO-ATC A16AA04
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
FDA ORPHAN DRUG 13986
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
EU-Orphan Drug EU/3/11/928
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
FDA ORPHAN DRUG 55690
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
FDA ORPHAN DRUG 262408
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FDA ORPHAN DRUG 258008
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
NDF-RT N0000187051
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NDF-RT N0000175549
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
WHO-VATC QA16AA04
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FDA ORPHAN DRUG 368712
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Code System Code Type Description
SMS_ID
100000085466
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
RXCUI
3022
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY RxNorm
NSC
647528
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
EVMPD
SUB08770MIG
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
NCI_THESAURUS
C61696
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
WIKIPEDIA
CYSTEAMINE
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
HSDB
7353
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
DRUG BANK
DB00847
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
CHEBI
17141
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PRIMARY
INN
195
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PRIMARY
DRUG CENTRAL
768
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PRIMARY
MESH
D003543
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PRIMARY
ECHA (EC/EINECS)
200-463-0
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
ChEMBL
CHEMBL602
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
PUBCHEM
6058
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
DAILYMED
5UX2SD1KE2
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
IUPHAR
7440
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
EPA CompTox
DTXSID3022875
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
CAS
60-23-1
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
FDA UNII
5UX2SD1KE2
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
USAN
CC-44
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
CHEBI
58029
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY
MERCK INDEX
m4046
Created by admin on Mon Mar 31 18:18:10 GMT 2025 , Edited by admin on Mon Mar 31 18:18:10 GMT 2025
PRIMARY Merck Index
Related Record Type Details
TARGET -> INHIBITOR
BINDER->LIGAND
BINDING
SALT/SOLVATE -> PARENT
SALT/SOLVATE -> PARENT
SALT/SOLVATE -> PARENT
Related Record Type Details
PRODRUG -> METABOLITE ACTIVE
Related Record Type Details
ACTIVE MOIETY
Name Property Type Amount Referenced Substance Defining Parameters References
Volume of Distribution PHARMACOKINETIC